Congenital Anomalies of the Esophagus Treatment & Management

To prevent mucous accumulation, aspiration, and respiratory deterioration continuous or intermittent low-pressure suction of the upper esophageal pouch should be initiated with a double-lumen Replogle catheter. In small infants, intermittent suction may be better. The infant should be positioned to minimize gastric fluid reflux. The infant is typically positioned in a 45 º sitting position. In addition, infant handling should be minimized because excess disturbance may lead to further respiratory complications, increased oxygen consumption, cold stress, and increased regurgitation of gastric contents. Oxygen therapy should be administered as needed to maintain oxygen saturation. Endotracheal intubation is not performed routinely, but it may be required based on the infant's respiratory status. Bag-mask ventilation should be avoided because it may cause gastric distension leading to increased reflux.

Intravenous fluid therapy consisting of 10% dextrose and hypotonic sodium chloride solution is used to maintain fluid, electrolyte, and glucose balance. Broad-spectrum antibiotics should be administered at the time of diagnosis or after cultures are obtained. A vitamin K analog should also be administered before surgery. Under no conditions should the infant be orally fed. If surgical treatment is delayed more than a few days, total parenteral nutrition is used. In addition, the infant should be transferred to a tertiary care pediatric institution with a neonatal ICU and a pediatric surgery team.

The operative procedure for infants with congenital esophageal abnormalities depends on the specific type of anomaly present, the condition of the infant, and the presence of other congenital anomalies.^{[4, 5]}

Gastrostomy

The staged approach for patients with pure esophageal atresia (EA) and in some infants with EA and tracheoesophageal fistula (TEF) includes initial placement of a Stamm gastrostomy, followed later by fistula division and later esophageal reconstruction. Gastrostomies may cause problems in infants with EA and TEF, especially in premature infants with severe respiratory distress syndrome requiring positive-pressure ventilation. Because of the TEF, the infant's respiratory and upper GI tracts function as a single unit. Therefore, the sudden decrease in intragastric pressure may result in preferential airflow through the fistula. Fistula ligation, occlusion of the fistula with a Fogarty catheter, and an underwater seal for the gastrostomy tube are methods used to maintain ventilatory pressure in these cases.

Most surgeons perform a gastrostomy in the first 24 hours of life in an infant with pure EA. This allows enteral feedings while the child grows and the esophageal gap shortens. A gastrostomy may be used in premature or unstable infants with EA and TEF.

Esophageal atresia with tracheoesophageal fistula

Fistula division with primary anastomosis is the surgical treatment for EA with TEF. A posterolateral thoracotomy on the side opposite the aortic arch is used. The patient is typically positioned on the left side with a small axillary roll. The right arm is extended above the head with the neck slightly flexed. Typically, manual ventilation control is used until the fistula is ligated.

For infants in whom ventilation is difficult because of the passage of air through the fistula into the stomach, insertion of a Foley catheter through the fistula into the lower esophagus may be helpful. This can be done through a bronchoscope or performed retrograde through the stomach accessed with a laparotomy incision. A fourth intercostal extrapleural approach is employed through a transverse incision along the inferior angle of the scapula, from the anterior axillary line posteriorly to the paravertebral region. Although some surgeons prefer the transpleural technique for its speed, the extrapleural approach provides added protection against empyema should an anastomotic leak occur. The latissimus dorsi is divided, and the serratus anterior can be divided or reflected to protect its innervation. A muscle-sparing approach can be performed.

The thorax is entered through the fourth intercostal space (ICS) by dividing the intercostal muscles. The extrapleural dissection begins posteriorly and proceeds superiorly, inferiorly, and finally anteriorly, where the risk of a pleural tear is highest. Wet cotton-tipped applicators, gauze swabs, moist peanut dissectors, or gentle finger dissection facilitates the pleural dissection from the chest wall. The azygous vein is identified, ligated and divided, or retracted (see image below). The lung and pleura are medially retracted. The distal esophageal segment is identified by following the right vagus nerve inferiorly, and the connection of the esophagus to the trachea is located. The lower esophagus is dissected circumferentially near the fistula taking care to not damage the vagal fibers or vascularization.

Extensive mobilization of the distal pouch is not recommended because of its segmental blood supply. However, distal pouch mobilization may be necessary to achieve a primary anastomosis. The fistula is divided close to the trachea. A 1-mm to 2-mm esophageal cuff should be left on the trachea to minimize the risk of postoperative tracheal stricture. Interrupted 5-0 or 6-0 silk or polypropylene sutures are used to close the fistula. The air-tightness of the tracheal closure should be assessed by filling the chest with saline and looking for any bubbles when positive-pressure is applied by the anesthesiologist. Atraumatic handling of the distal esophagus is important. Two fine-stay sutures in the distal esophagus allow for mobilization and gentle traction without the use of forceps.

The proximal segment is identified by gently advancing the Replogle tube, and traction sutures may be placed. The upper pouch can and should be mobilized all the way to the thoracic inlet. Circumferential mobilization to the thoracic inlet aids in identifying proximal fistulas. Care must be taken during the dissection of the proximal esophagus to avoid injury to the trachea or recurrent laryngeal nerve.

A primary anastomosis should be performed whenever possible. If the esophageal segments cannot be joined without undue tension after mobilization of both ends, a circular myotomy may be performed on the proximal segment. A primary anastomosis is accomplished by opening the proximal pouch at the lower-most point. The opening created in the upper pouch should approximate that of the lower pouch. The distal segment is incised only if it is clearly devascularized or narrow and fibrous. Interrupted absorbable sutures are used to perform the anastomosis. Lateral stay sutures are placed. Ensuring mucosal apposition during the anastomosis is important. Usually, 5-6 sutures are needed to complete the posterior row. After all posterior sutures have been placed, the knots are tied on the inside of the esophageal lumen to prevent subsequent twisting of the esophagus. As the sutures are tied, tension gradually is distributed to the tied sutures. The lateral stay sutures are not tied.

A small feeding tube can be passed across the anastomosis into the stomach to ensure luminal patency and protect the posterior wall of the anastomosis while the anterior wall is sutured. Some surgeons leave the tube in postoperatively to decompress the stomach and provide a postoperative feeding route (see image below).

The anterior suture layer of the anastomosis is then completed over the tube, and the knots are tied on the outside (see image below). A muscle or tissue flap may also be placed between the anastomosis and the repaired trachea to decrease the risk of fistula recurrence.

Most surgeons place a retropleural chest tube with the tip positioned near, but not touching, the anastomosis (see image below). The tube is placed to water seal drainage to avoid an extrapleural pneumothorax. Suction applied to the tube may disrupt the anastomosis. Intubation should be maintained as is clinically required. Premature extubation that results in aggressive bag-mask ventilation and reintubation can be disastrous to the repair.

Premature and medically unstable children can be maintained on total parenteral nutrition with a tube in the proximal pouch until surgery. Emergent thoracotomy and fistula ligation is used for infants with respiratory distress when nonoperative management is unsuccessful.

Recently, thoracoscopic techniques have been used to repair tracheoesophageal atresia and distal fistula.

Esophageal atresia with proximal fistula

The repair for a proximal fistula is the same as that described above. If a proximal fistula is present with no distal fistula, a cervical approach may be used instead.

Pure esophageal atresia

Most children with pure EA undergo gastrostomy placement followed by delayed repair. Following a period of growth, repair of EA without fistula consists of a thoracotomy with retropleural dissection, mobilization of the 2 esophageal segments, and a primary anastomosis (see images below). Esophageal continuity can usually be achieved without an esophageal replacement.

Several techniques are used to lengthen the esophageal ends to achieve a primary anastomosis. Bougienage is the most common mechanical lengthening procedure. Upper pouch bougienage is performed by passing a weighted bougie through the mouth into the upper pouch and applying forward pressure 1-2 times daily. This procedure is performed for 6-12 weeks followed by a delayed primary repair. Both internal and external traction sutures have been used by some surgeons.

During surgery, lengthening techniques may also be used.^{[6, 7]} Myotomy is a common method that provides a 1-cm increase in length. The muscular layers are divided to create a plane between the muscularis propria and submucosa. Circular myotomies involve a circumferential division, while spiral myotomies preserve the muscular continuity of the proximal pouch and maintain closed submucosal layers. As many as 3 myotomies can be performed. The most proximal myotomy should be performed first. This procedure can be performed on both esophageal segments, but myotomies performed on the distal stump may increase the incidence of gastroesophageal reflux.

When additional length is necessary, a portion of the stomach may be brought up through the diaphragmatic hiatus. This procedure has been successfully used, even in infants with low birth weight. A Collis lengthening procedure may also be used.

A cervical esophagostomy is used when an anastomosis is impossible and in cases of failed surgery. For this procedure, a left transverse incision is performed 1 cm above and parallel to the medial third of the clavicle. The incision is deepened through the platysma. The sternal head of the sternocleidomastoid is divided, the sternothyroid muscle is divided or reflected, and the carotid sheath is retracted. The esophagus is mobilized circumferentially and dissected distally. The esophageal end is brought out to the lateral end of the skin incision and sutured with interrupted absorbable sutures. This procedure allows the child to swallow normally. Sham feeds are administered to stimulate lengthening by a natural bougienage effect and to avoid oral aversion. The infant may be discharged home on gastrostomy feedings until esophageal repair or replacement can be performed.

A variant of this method is the multistaged extrathoracic elongation, which has also been used in long-gap treatment. In this procedure, the upper esophagus is initially mobilized and brought out as an end cervical esophagostomy. Over a period of weeks to months, the esophagus and stoma are progressively translocated down the anterior chest until adequate length is achieved to permit an end-to-end anastomosis. Again, the child is able to swallow and can be discharged home during the intervening period. The use of short-term traction sutures to narrow the esophageal gap has been described.

Esophageal replacement

Esophageal substitutions are used to restore esophageal continuity when the patient's native esophagus is not an option. No esophageal replacement is ideal, and a poorly functioning esophagus may even be preferable to any esophageal substitute. Because of advancements in the surgical techniques to treat EA, the need for esophageal replacement has diminished; however, conditions that necessitate an esophageal substitution are noted.

Many types of conduits have been used, including colon, stomach, gastric tubes, and jejunum. The choice of substitute to be used is influenced by the length and segment of esophagus to be replaced, the presence of any associated anomalies, and the vascular adequacy of the proposed replacement. Most often, the surgeon's preference and experience contributes to the selection.

• Colon

  • Segments of the right, left, or transverse colon may be used (see image below). The colon interposition may be placed in a posterior mediastinal or retrosternal position within the thorax. Colonic interpositions act as passive conduits, allowing isoperistaltic and antiperistaltic positions within the chest. Reports of spasm, regurgitation, and discomfort with the antiperistaltic colonic conduits indicate some retention of motor function. The isoperistaltic method is often favored. Contrast study demonstrating colonic interposition. The colon is sutured to the cervical esophagus proximally and the stomach distally. No leak or stricture is noted.
  • Advantages of colonic replacement of the esophagus include the following:
    • Adequate length available
    • Acts as a conduit antiperistaltically or isoperistaltically
    • Good vascular supply via marginal artery
    • Conserves native stomach
    • Can be placed in esophageal bed of posterior mediastinum
    • Has memory (but variable) for propelling solid bolus
    • Has mucous shield, which protects against reflux
    • Responds to acid with a peristaltic rush for clearance
    • May allow preservation of entire native esophagus
    • Minimizes/eliminates tension on the upper and lower esophageal segments
    • May improve esophageal motility and minimize reflux
  • Disadvantages of colonic replacement of the esophagus include the following:
    • Requires 3 anastomoses
    • Empties more slowly than the esophagus
    • Requires preoperative bowel preparation
    • Long surgical procedure with extensive mobilization
    • Dilates and becomes redundant over time
    • Slows food transit
    • Anastomotic leak and stricture
    • Graft necrosis
    • Long-term growth retardation
• Gastric tube

  • Esophageal replacement can be achieved by fashioning a tube from the stomach. The 2 most common tubes are the reversed (antiperistaltic) tube and the nonreversed (isoperistaltic) tube. Delaying the procedure allows stomach enlargement, and a long tube can then be constructed. Gastrostomy tubes should be placed close to the lesser curvature of the stomach in these infants.
  • The reversed gastric tube is constructed most commonly. It is proximally based and supplied by the left gastroepiploic vessels. This tube can be used to replace the entire esophagus but has a more limited blood supply than the isoperistaltic tube, and care must be taken during the procedure not to injure the spleen. The nonreversed, or isoperistaltic, tube is based distally and supplied by the right gastroepiploic vessels. A 1-stage or 2-stage procedure may be performed. A staged procedure involves constructing the gastric tube and externalizing it in the neck. A cervical anastomosis is completed 4-6 weeks later. The gastric tube and gastric remnant should be studied radiographically before reanastomosis.
  • Advantages of a gastric tube include the following:
    • Thick-walled straight conduit
    • Does not become dilated, tortuous, or redundant
    • Less risk of ischemia because of robust blood supply
    • Simplified construction through stapler use
    • Has a favorable anatomic location in the upper abdomen
    • Possible construction variations
    • Has adequate length
    • Requires fewer anastomoses
    • Has comparable diameter and occupies less space in the thorax and neck
    • Has only one suture line
    • Requires no bowel preparation and is a faster procedure
    • Rapidly transits
  • Disadvantages of a gastric tube include the following:
    • Leaks and strictures
    • Extensive gastroesophageal reflux, which may lead to peptic ulceration, nocturnal coughing, and Barrett epithelium
    • Leaves a small gastric reservoir
    • Creates a long suture line
    • May result in gastric outlet obstruction
    • May be unable to reach high in neck
    • Difficult to place in posterior mediastinum
    • Occasional perforation
• Gastric transposition

  • In a gastric transposition, the entire stomach assumes an intrathoracic position and serves as a passive conduit (see image below). The fundus of the stomach is joined to the upper esophageal pouch, and the lower esophageal segment is not used. A gastric transposition can be performed in infants and children of all ages but is typically performed around the first year of life. Although some prefer this method of esophageal replacement, others advise against its use in infants and children because of the effects of the gastric capacity crowding the lungs and trachea and the excessive gastroesophageal reflux. An initially placed gastrostomy is not a contraindication. Contrast swallow (oblique view) demonstrating a gastric pull-up used as esophageal replacement in a child with pure esophageal atresia. Note stomach in chest.
  • Advantages of gastric transposition include the following:
    • Readily available and easily mobilized stomach
    • Involves single anastomosis
    • Adequate length available
    • Can use numerous surgical approaches
    • Has excellent blood supply
    • Involves a technically easy procedure
    • Has low incidence of leaks and strictures
  • Disadvantages of gastric transposition include the following:
    • Large bulk possibly causing space problems intrathoracically
    • Reflux
    • Barrett epithelium development possible in proximal esophagus
    • Possible stricture or aspiration due to lack of gastroesophageal valve
    • Poor gastric emptying
    • May affect pulmonary function
    • Results in depleted iron stores causing anemia
    • May affect growth
    • Microvasculature easily disturbed with rough handling
    • May not reach as high in neck as other methods because of blood supply
    • Difficult to initiate oral feedings
    • May result in dumping as a common postoperative symptom
• Jejunum

  • Jejunal esophageal substitution is rarely used. The jejunal replacement is unique because it retains peristaltic activity and, therefore, must always be positioned isoperistaltically. However, peristalsis is not synchronous with swallowing and may slow food transit. Proximal jejunal segments are preferred for graft construction because of their larger and fewer vascular arcades, which can be located and dissected more easily. Jejunal segments may range from 2-12 cm in length, but the tight radii of the superior mesenteric artery branches prevent construction of straight and longer segments. The jejunum is typically used only for replacements of short lower esophageal lengths.
  • Advantages of jejunal replacement of the esophagus include the following:
    • A remarkably disease-free organ
    • Readily available
    • Jejunal caliber similar to that of normal esophagus
    • Functions as reliable food transporter
    • Results in low incidence of leaks and strictures
    • Functions as an effective gastroesophageal barrier
    • Does not require a bowel preparation
    • Jejunal wall at correct thickness for comfortable suture placement
  • Disadvantages of jejunal replacement of the esophagus include the following:
    • Length of conduit limited by blood supply
    • Infarction commonly resulting from passage through chest
    • Procedure more technically difficult
    • Requires 3 anastomoses
    • Has high peptic ulcer susceptibility
    • Blood supply lacking marginal artery
    • Difficult to obtain a straight conduit with sufficient vascularization
    • Has high failure rate (free graft)

Isolated tracheoesophageal fistula

H-fistulas or N-fistulas are typically located at the T1-T3 level, coursing downward from the trachea to the esophagus. Most can be repaired through a cervical approach. An undiagnosed H-type fistula may also be identified while mobilizing the proximal esophagus during surgery for EA (see image below). A right-sided supraclavicular incision 1-1.5 cm above and parallel to the right clavicle is made to minimize risk of injury to the thoracic duct. The sternocleidomastoid muscle is retracted posteriorly, and the sternal head is divided if necessary. The inferior thyroid artery and middle thyroid vein are divided if needed to expose the plane between the trachea and the esophagus.

Care should be taken to clearly identify and preserve the recurrent laryngeal nerves, vagal nerve fibers, and posterior trachea. The fistula may be located higher than might be expected. The esophagus is encircled with rubber vessel loops to facilitate mobilization. The fistula is also encircled when it is identified (see image above). The fistula is divided close to the esophagus, leaving a 2-mm esophageal cuff on the trachea. Interrupted sutures are used to close the esophagus and trachea (see image below).

A muscle flap may be interposed between the 2 suture lines to decrease the likelihood of a recurrent fistula. Fistula ligation without division should not be performed. Wound drainage is typically not necessary for an H-fistula repair, and the endotracheal tube should be left in because tracheal swelling is a frequent postoperative occurrence.

Congenital esophageal stenosis, webs, and tracheobronchial remnants

Treatment should relieve the obstructive symptoms and maintain the antireflux mechanism of the gastroesophageal junction. Bougienage or balloon dilation may successfully treat fibromuscular hypertrophy. Membranous webs, complete occlusion, and tracheobronchial remnants usually require surgical excision (see images below). A right thoracotomy is usually used. Lesions in the abdominal esophagus can be approached through the abdomen. A segmental resection and primary anastomosis can be achieved in most instances (see images below). The phrenic and vagus nerves should be identified and preserved. Esophageal replacement may be needed for long segments of fibromuscular hypertrophy.

Postoperative care of an infant with EA involves a team approach, typically in the neonatal intensive care unit. A chest radiograph is immediately obtained following surgery (see images below). The infant should be kept in a semiupright position, and the patient's head should be supported to prevent neck extension, which may disrupt the anastomosis. Intravenous fluid replacement should be maintained, and prophylactic antibiotic treatment should be continued. The pharynx should be frequently suctioned to prevent respiratory infection, but deep suctioning should be avoided. Appropriate temperature, humidity, and oxygen atmospheric control are essential. If the anastomosis was performed under extensive tension, some surgeons recommend elective paralysis and mechanical ventilation for several days postoperatively. Otherwise, the patient is weaned from the ventilator as soon as possible.

Contrast esophagraphy is performed postoperatively to assess for esophageal leak, stricture, motility, and gastroesophageal reflux (see image below). The swallowing reflex and positions of the duodenum and ligament of Treitz should also be examined. The timing of the initiation of feeding varies. Some advocate starting gastrostomy or nasogastric feedings on the first or second postoperative day in uncomplicated cases. Other surgeons advise against gastrostomy or nasogastric tube feedings because of the potential for acid reflux and injury to the anastomosis. Total parenteral nutrition should be used when enteral feedings are not started. If no leaks are observed on the postoperative contrast study, feedings are initiated and the chest tube is removed. The child may be discharged when feedings are tolerated and appropriate weight gain is observed.

Frequent follow-up visits are necessary during the first year after repair. If the child is doing well, visits can be decreased to 1-2 times per year until school age. Because of scarring at the anastomosis, the child may tolerate only pureed food up to age 12-18 months and then minced food until age 5 years. At age 5 years, the child has typically learned to chew well before swallowing and has developed sufficient teeth to aid in this task. The child's parents should be informed about the signs of gastroesophageal reflux, recurrent fistula, tracheomalacia, and other complications.

The severity of complications following esophageal surgery is often dictated by the extent of the repair. Anastomotic tension is involved in 79% of complications, and the most common complications include anastomotic leak, recurrent fistula, stricture, and gastroesophageal reflux.

Anastomotic leakage occurs in anywhere from 14-21% of children that have undergone a surgical esophageal atresia (EA) repair (see following image). Leaks result from the small friable lower segment, ischemia of the esophageal ends, excess anastomotic tension, sepsis, poor suturing techniques, and inaccurate mucosal apposition.

Early extubation with reintubation also puts infants at increased risk of anastomotic leakage. Most leaks are small, occur late after the first 48 hours, and require only conservative management. Chest tube drainage, antibiotics, and time allow most to heal. Spontaneous healing occurs in 95% of leaks when a mediastinal drain is present. A repeat esophagraphy is performed each week until the leak has resolved. More significant leaks occur early, within the first few days, and should be immediately explored in most cases. Major anastomotic disruptions account for only 3-5% of leaks. Large leaks can be fatal or may lead to fistula recurrence.

Fistula recurrence is observed in 3-14% of patients treated for EA-TEF or isolated TEF. Fistulas usually recur within a few months but may be found as late as 2 years postoperatively. Fistula recurrence is caused by anastomotic leak with local inflammation and erosion at the previous repair site, ischemia, and surgical dissection too near the trachea. Recurrent TEF should be suspected when choking episodes occur during feeding and/or recurrent pneumonia is observed. Esophagography under video fluoroscopy with the patient in the prone position or bronchoscopy provide the best methods of diagnosis. Routine contrast swallows do not reveal 50% of recurrent TEFs. Fistulas do not spontaneously close and require surgical division and suturing.

Recently, attempts have been made to close recurrent fistulas with fibrin glue administered into the fistula. During surgical repair, a tissue flap should be interposed between the suture lines of the trachea and esophagus. Recurrence rates remain in the 10-20% range when repeat surgery is needed.

Esophageal strictures are also common following esophageal surgery. Anastomotic strictures occur in as many as 40% of children with an EA repair (see following images). Strictures can result from the natural healing process, the different sizes of the 2 anastomosed segments, tension, gastroesophageal reflux, and leaks. Asymptomatic narrowing observed on initial esophagography can improve over time without the need for intervention. Strictures are clinically suspected, and the diagnosis can be confirmed via contrast esophagraphy and esophagoscopy.

Most strictures can be managed with serial dilatations. Multiple dilatations over several months are needed in many cases. Strictures unresponsive to dilation require surgical resection.

Gastroesophageal reflux is a common complication of esophageal surgery, occurring in 40-70% of patients undergoing EA repair. Symptoms of gastroesophageal reflux include coughing, apnea, recurrent pneumonia, failure to thrive, and stricture formation (see following images). Reflux is thought to be related to tension, dysmotility of the lower esophagus, and an altered angle of His from distal esophageal mobilization.

Gastroesophageal reflux is clinically diagnosed and may be confirmed with an upper GI series or pH probe. Reflux is initially medically managed. Initial steps include keeping the patient in a prone head-up position after feeding, thickening feeds, and giving smaller more frequent meals. Acid reduction agents (eg, histamine H2 receptor blockers, proton pump inhibitors) and prokinetic agents may be needed. If symptomatic reflux persists, a fundoplication is needed. Fundoplications are required in up to one half of patients with gastroesophageal reflux after an EA repair.

Tracheomalacia is a condition in which weakness of the trachea results in compression of the anterior and posterior walls between the aorta and dilated esophagus during expiration or coughing. This complication occurs more frequently in the presence of a fistula and is present in 10-20% of infants after an EA-TEF repair. The region of compression is typically located at or just above the level of the original fistula but may involve the entire trachea. The marked tracheal anterior-posterior collapse is observed easily during bronchoscopy performed while the patient is awake. Tracheomalacia usually improves slowly with time. In some cases, tracheomalacia may prevent extubation, and intervention with aortopexy, tracheostomy, or tracheal stenting is needed.

Esophageal dysmotility is frequent following surgery for congenital esophageal lesions. Food impaction can occur at the level of the anastomosis, especially if a stricture is present (see following image). Altered esophageal peristalsis has been documented with manometric and radionuclide studies, video fluoroscopy, scintigraphy, and cine esophagraphy after EA repair. Discontinuity of peristaltic function is observed above and below the surgical anastomosis. Dysmotility appears to persist and has been reported in 32-year follow-up studies. Children learn to compensate for the dysmotility by eating in an upright position and drinking frequently during eating.

Esophageal diverticula may develop at the anastomosis or a site where a circular myotomy was performed. The myotomy site may balloon progressively over time and cause ventilatory obstruction and dysphagia.

The survival rate of patients with esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) has immensely improved since Haight's first successful repair in 1941. Early diagnosis and advancements in neonatal anesthesia, surgical technique, treatment of associated anomalies, and intensive care management have improved the prognosis. Most children treated for EA have a normal lifespan. Despite an increased number of patients with severe congenital anomalies, survival rates have been reported as high as 95%. In uncomplicated cases, survival rates are virtually 100%.

Traditionally, prognosis for children with EA-TEF was based on birth weight and the presence of pneumonia and associated congenital anomalies. Because of advancements in neonatal care, birth weight does not affect survival rate unless it is severely low, and pneumonia may be treated successfully. Currently, cardiac and chromosomal abnormalities are the most significant causes of death. Infants with a birth weight less than 1500 g, major congenital cardiac abnormalities, severe associated anomalies, preoperative ventilator dependence, and/or long gap are at increased risk.

Dysphagia, frequent night coughs, dyspepsia, and recurrent respiratory infections are frequent results of the less distensible esophagus and gastroesophageal reflux. Gastroesophageal reflux occurs in as many as one half of these patients and many require antireflux operations. Feeding difficulties also are common, particularly during the first several years after repair. Choking, vomiting, and food impaction occur. These symptoms, like many following EA repair, diminish over time, and 70-80% of adolescents report no or only occasional swallowing impairment. Most patients who have undergone EA repair have abnormal peristalsis with decreased contractile activity and inefficient clearance capacities.

In one series, after an average of 8.8 years of follow-up care, all patients were reported to eat excellently or satisfactorily, with more than 90% eating no differently than their siblings. Normal respiratory function is observed in half of patients 3 months postoperatively. Tracheomalacia, vascular rings, and decreased lung volumes account for the abnormal respiratory function in the other children. Tracheomalacia occurs in 10% of patients with TEF. Most outgrow this problem; however, some children require more aggressive therapy.

Growth retardation has been observed in some children who have had EA repair, but this observation varies. Patients treated for EA-TEF are at higher risk for developing esophagitis and Barrett epithelium. Reports of esophageal carcinoma decades after EA-TEF repair are becoming more frequent as the first generation of survivors progresses through adulthood. Surveillance esophagoscopy has been proposed to provide early detection for esophageal abnormalities.

Despite the complications, the results of EA-TEF repair have dramatically improved. Many symptoms are alleviated over time, and most children and adults enjoy normal lifestyles and have no complaints concerning their quality of life or eating habits. Even by school age, children who had many complications in infancy reported few restrictions at school or in participation in sports with little or no effect on school attendance and social activities. The outcome for these children and children treated for other congenital lesions is generally good.

The prognosis and treatment course for infants with esophageal atresia (EA) and/or tracheoesophageal fistula (TEF) and other congenital lesions has improved over the past 60 years. Advances in perinatal and neonatal care have been paramount in reducing the morbidity and mortality rates associated with these conditions. Currently, associated congenital anomalies and pulmonary complications contribute most significantly to adverse outcomes.

Infants with very low birth weight or serious cardiac abnormalities are at increased risk for poor outcome. Improvements in the prevention and management of these high-risk infants would improve outcome and survival rates. In addition, enhanced prenatal detection of EA and/or TEF and other congenital anomalies allows for better prenatal counseling and preparation for the delivery at a tertiary medical center.

Esophageal defects are currently repaired with thoracoscopy; robotic-assisted surgery may be used in the future.^[8]

Tissue engineering for esophageal replacement, in utero intervention, and minimally invasive techniques such as thoracoscopy and robotic assistance may be used in years to come to further improve treatment of these infants.